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Literature DB >> 7668247

Very-long-chain acyl-CoA dehydrogenase deficiency: molecular genetics of a mitochondrial membrane enzyme.

P M Coates.

Abstract

Entities: Disease

Mesh：

Substances：

Year: 1995 PMID： 7668247 PMCID： PMC1801537

Source DB: PubMed Journal: Am J Hum Genet ISSN： 0002-9297 Impact factor: 11.025

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12 in total

1. Novel fatty acid beta-oxidation enzymes in rat liver mitochondria. II. Purification and properties of enoyl-coenzyme A (CoA) hydratase/3-hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase trifunctional protein.

Authors: Y Uchida; K Izai; T Orii; T Hashimoto
Journal: J Biol Chem Date: 1992-01-15 Impact factor: 5.157

2. Rat very-long-chain acyl-CoA dehydrogenase, a novel mitochondrial acyl-CoA dehydrogenase gene product, is a rate-limiting enzyme in long-chain fatty acid beta-oxidation system. cDNA and deduced amino acid sequence and distinct specificities of the cDNA-expressed protein.

Authors: T Aoyama; I Ueno; T Kamijo; T Hashimoto
Journal: J Biol Chem Date: 1994-07-22 Impact factor: 5.157

3. A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase.

Authors: T Aoyama; Y Uchida; R I Kelley; M Marble; K Hofman; J H Tonsgard; W J Rhead; T Hashimoto
Journal: Biochem Biophys Res Commun Date: 1993-03-31 Impact factor: 3.575

4. Molecular cloning and nucleotide sequence of cDNAs encoding human long-chain acyl-CoA dehydrogenase and assignment of the location of its gene (ACADL) to chromosome 2.

Authors: Y Indo; T Yang-Feng; R Glassberg; K Tanaka
Journal: Genomics Date: 1991-11 Impact factor: 5.736

5. Immunochemical characterization of variant long-chain acyl-CoA dehydrogenase in cultured fibroblasts from nine patients with long-chain acyl-CoA dehydrogenase deficiency.

Authors: Y Indo; P M Coates; D E Hale; K Tanaka
Journal: Pediatr Res Date: 1991-09 Impact factor: 3.756

6. Novel fatty acid beta-oxidation enzymes in rat liver mitochondria. I. Purification and properties of very-long-chain acyl-coenzyme A dehydrogenase.

Authors: K Izai; Y Uchida; T Orii; S Yamamoto; T Hashimoto
Journal: J Biol Chem Date: 1992-01-15 Impact factor: 5.157

7. Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the alpha-subunit of the mitochondrial trifunctional protein.

Authors: L IJlst; R J Wanders; S Ushikubo; T Kamijo; T Hashimoto
Journal: Biochim Biophys Acta Date: 1994-12-08

Very-long-chain acyl-CoA dehydrogenase deficiency: molecular genetics of a mitochondrial membrane enzyme.

1. Novel fatty acid beta-oxidation enzymes in rat liver mitochondria. II. Purification and properties of enoyl-coenzyme A (CoA) hydratase/3-hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase trifunctional protein.

2. Rat very-long-chain acyl-CoA dehydrogenase, a novel mitochondrial acyl-CoA dehydrogenase gene product, is a rate-limiting enzyme in long-chain fatty acid beta-oxidation system. cDNA and deduced amino acid sequence and distinct specificities of the cDNA-expressed protein.

3. A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase.

4. Molecular cloning and nucleotide sequence of cDNAs encoding human long-chain acyl-CoA dehydrogenase and assignment of the location of its gene (ACADL) to chromosome 2.

5. Immunochemical characterization of variant long-chain acyl-CoA dehydrogenase in cultured fibroblasts from nine patients with long-chain acyl-CoA dehydrogenase deficiency.

6. Novel fatty acid beta-oxidation enzymes in rat liver mitochondria. I. Purification and properties of very-long-chain acyl-coenzyme A dehydrogenase.

7. Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the alpha-subunit of the mitochondrial trifunctional protein.

8. Analysis of fatty acid oxidation intermediates in cultured fibroblasts to detect mitochondrial oxidation disorders.

9. Two alpha subunit donor splice site mutations cause human trifunctional protein deficiency.

10. Long-chain acyl coenzyme A dehydrogenase deficiency: an inherited cause of nonketotic hypoglycemia.

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